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Industry: Email Alert RSS FeedDelayed endolymphatic hydrops: Study and review of clinical implications and surgical treatment - Brief Article
Ear, Nose & Throat Journal, Feb, 2001 by Tsun-Sheng Huang, Ching-Chen Lin
Her tympanic membranes were normal, and the rest of her physical examination was unremarkable. A preoperative audiogram showed 25 dB PTA and a 100% SDS in the right ear and confirmed her total deafness in the left. Her blood test results were unremarkable, and her mastoid x-ray was normal on both sides. A glycerol test was positive on the right side. Because her vertigo had become incapacitating, ELS surgery was performed on the right ear with an Arenberg's inner ear valve in 1984. Following surgery, the patient's hearing increased by 10 dB PTA, and to date her vertigo is still completely resolved.
Discussion
Symptomatology. Schuknecht and Gulya described endolymphatic hydrops in terms of its symptomatic and asymptomatic forms. [7] DEH can be considered an asymptomatic, clinically silent type because a unilateral deafness caused by an earlier otologic insult can exist for years before it becomes symptomatic. Meanwhile, Meniere's disease is defined as the idiopathic, symptomatic form of endolymphatic hydrops of unknown cause because symptoms develop in one or both ears that had previously had normal auditory and vestibular functions. This acquired form of endolymphatic hydrops or Meniere's syndrome includes cases in which a documented insult has been caused by either inflammation or trauma to a previously normal labyrinth.
Ipsilateral DEH is characterized by symptoms that mimic those of Meniere's-type vertigo. These symptoms occur after a lapse of several to many years following the onset of deafness in that ear. [1,2] Symptoms of contralateral DEH, in which a Meniere's symptom complex develops in the ear opposite the deaf ear, typically include progressive and fluctuating hearing loss, aural pressure and tinnitus, and/or episodic attacks of vertigo accompanied by nausea and vomiting. In bilateral DEH, a profound hearing loss in both ears precedes the onset of symptoms.
It has been proposed that DEH is a pathologic condition that is the final common manifestation of a variety of otologic insults, including infection (either viral or bacterial), trauma (either physical or acoustic), the onset of sudden deafness, and other causes known and unknown. [7] Schuknecht suggested that in DEH, the reabsorption of endolymph is compromised as a result of two possible consequences of the original insult: either damage to the endolymphatic sac or blockage of the vestibular aqueduct. [3] If so, this implies that some auditory and/or vestibular function remains because some secretory tissue is still present, although many patients eventually develop cochleovestibular symptoms.
Schuknecht and Gulya later described two unique temporal bone histopathologic studies in two patients with contralateral DEH. [7] They found that the histopathology in the deafened ear was typical of viral labyrinthitis (based on its similarity to the histopathology of ears deafened by mumps or measles) and that pathologic changes in the opposite ear were similar to those seen in Meniere's disease. [8] Supplementing their findings with those of Koga et al [9] and Westmore et al, [10] who suggested that unilateral hearing loss in childhood is often caused by subclinical mumps, Schuknecht et al [4] proposed that patients with contralateral DEH probably experienced a viral attack in the hearing ear at the same time the opposite ear was deafened.
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